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Videokeratographic abnormalities in a family with posterior polymorphous dystrophy
1Department of Ophthalmology, University of Louisville, Kentucky 40202, USA.
Cornea
|July 24, 1998
Summary
This study found that a specific videokeratographic pattern of steep corneas is linked to posterior polymorphous dystrophy (PPD) in one family. This finding highlights a potential diagnostic marker for this genetic eye condition.
Area of Science:
- Ophthalmology
- Genetics
- Corneal Diseases
Background:
- Posterior polymorphous dystrophy (PPD) is a rare, inherited corneal disease.
- The vesicular form of PPD has a distinct genetic basis, often autosomal dominant.
- Accurate diagnosis and understanding of PPD phenotypes are crucial for patient management.
Observation:
- A descriptive case series involving five family members with PPD was conducted.
- Detailed ophthalmic examinations included keratometry, videokeratography, axial length, and pachymetry.
- A consistent and unique videokeratographic pattern was observed across affected individuals.
Findings:
- All PPD-affected family members exhibited significantly steep corneas on videokeratography.
- Keratometry readings were more than three standard deviations above the reported mean for healthy individuals.
- This pronounced corneal steepening represents a unique videokeratographic signature.
Implications:
- Videokeratographic corneal steepening may serve as a characteristic indicator for PPD.
- This finding could aid in the early diagnosis of autosomal dominant vesicular PPD.
- Further research into this videokeratographic pattern may reveal more about PPD pathogenesis.